PLATELETS AND THROMBOPOIESIS Dysmegakaryopoiesis of FPD/AML pedigrees with constitutional RUNX1 mutations is linked to myosin II deregulated expression
نویسندگان
چکیده
1Institut National de la Santé et de la Recherche Médicale, Villejuif, France; 2Institut Gustave Roussy, Villejuif, France; 3Université Paris Sud, Villejuif, France; 4Instituto de Investigaciones Médicas Alfredo Lanari, Universidad de Buenos Aires, Consejo Nacional de Investigaciones Cientificas y Technicas, Buenos Aires, Argentina; 5Centre de Référence des Pathologies Plaquettaires, Hôpital Haut-Lévêque, Pessac, France; 6Institut National de la Santé et de la Recherche Médicale, Faculté de Médecine La Timone, Marseille, France; and 7Assistance Publique–Hôpitaux de Paris, Hôpital Trousseau, Service d’Hématologie biologique, Paris, France
منابع مشابه
Regulation of platelet myosin light chain (MYL9) by RUNX1: implications for thrombocytopenia and platelet dysfunction in RUNX1 haplodeficiency.
Mutations in transcription factor RUNX1 are associated with familial platelet disorder, thrombocytopenia, and predisposition to leukemia. We have described a patient with thrombocytopenia and impaired agonist-induced platelet aggregation, secretion, and glycoprotein (GP) IIb-IIIa activation, associated with a RUNX1 mutation. Platelet myosin light chain (MLC) phosphorylation and transcript level...
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Haploinsufficiency of RUNX1 (also known as CBFA2/AML1) is associated with familial thrombocytopenia, platelet dysfunction, and predisposition to acute leukemia. We have reported on a patient with thrombocytopenia and impaired agonistinduced aggregation, secretion, and protein phosphorylation associated with a RUNX1 mutation. Expression profiling of platelets revealed approximately 5-fold decrea...
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• Myosin-II inhibition (with blebbistatin) and MYH9-RD mutations enhance shear fragmentation to pre/ proplatelet sizes. • Sustained shear activates normal myosin-II, which then favors division of pre/ proplatelets to smaller platelets. Megakaryocyte ploidy and the generation of pre/proplatelets are both increased in culture by pharmacologic inhibition of myosin-II, but nonmuscle myosin-IIA (MII...
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Reticulated platelets with a high RNA content represent the most recently released platelets and are regarded to reflect thrombopoiesis. In the present study we used flow cytometric analysis to determine the percentage of reticulated platelets in peripheral blood for patients with chronic myeloproliferative disorders (polycythemia vera, essential thrombocytosis) and acute myelogenous leukemia (...
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